Poster presentation
Name of the presenting author - Dr. K.Achyuth,DNB resident,KIMS
Secunderabad.
Co-authors - Dr. Deepika and Dr. Siddhardha. KIMS Secunderabad.
TITLE-CNS RELAPSE IN ACUTE PROMYELOCYTIC LEUKEMIA.
Introduction-Acute promyelocytic leukemia(APL) is a highly curable disease
in the era of all‐trans retinoic acid (ATRA) but still approximately 10% to 20%
of cases relapse[1].When there is central nervous system (CNS) involvement,
eradication of abnormal promyelocytes from the patients is difficult. As a result,
median overall survival is shorter in patients with CNS involvement than that in
patients with haematological recurrences only[1].
Case report
Case 1-A 10-year old girl presented with complaints of purpuric rash over the
body and gum bleeding since 2 days. Hemogram showed Hb 9.3gm/dl, WBC
4,900cells/cumm (84% abnormal promyelocytes with neutropenia) and platelet
13,000/cumm. t(15:17) translocation with additional 21 translocation was
confirmed by FISH. She was discharged with stable counts after ATRA therapy
and was on follow up.
After two years she presented with c/o headache, fever, weakness of both left
upper and lower limb. CSF cytology – Positive for atypical cells, CSF sample
sent for FISH analysis - PML- RARA was detected.
Case 2–A-11 years old male child came with complaints of fever since 10 days.
Hemogram showed Hb 7.7 gm/dl, WBC 1,12,140 cells/cumm(84% abnormal
promyelocytes) and platelet 26,000/cumm. t(15:17) translocation was confirmed
by FISH. He was discharged with stable counts after ATRA therapy and was on
follow up.
After 2 months he presented with chief c/o of seizures and drowsiness. MRI
showed leptomeningeal metastasis, Csf cytology-atypical cells (100%)
Impression-positive for haematological malignancy, In bone marrow sample -
PML RARA detected.
Conclusion - Central nervous system (CNS) involvement is a rare complication
of acute promyelocytic leukemia (APML) which is encountered more
frequently at the relapse than at presentation. The incidence of CNS relapse
ranges between 0.6% and 2% [2]. Two cases of CNS relapsed APML at KIMS
are presented.
References
1. Furuya A, Kawahara M, Kumode M, Ohira Y, Usui A, Nagai S, Hosoba
S, Minamiguchi H, Kito K, Andoh A. Central nervous system
involvement of acute promyelocytic leukemia, three case reports. Clin
Case Rep. 2017 Mar 29;5(5):645-653. doi: 10.1002/ccr3.919. PMID:
28469869; PMCID: PMC5412762.
2. Ji M, Chi HS, Jang S, Park CJ, Lee JH, Seo JJ. Acute promyelocytic
leukemia presenting with central nervous system involvement: a report of
2 cases. Korean J Lab Med. 2011 Jan;31(1):9-12. doi:
10.3343/kjlm.2011.31.1.9. PMID: 21239864; PMCID: PMC3111037.
